特发性血小板减少性紫癜
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DISORDERS OF HEMOSTASIS
Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU
General Considerations
• Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation)
IDIOPATHIC (AUTOIMMUNE) THROMBOCYTOPENIC PURPURA (ITP)
General Considerations
• ITP is an autoimmune disorder in which an IgG autoantibody is formed that binds to platelets
（2）more delicate capillary wall
Clinical Findings
• Symptoms and Signs: • occurs ：
in childhood, frequently precipitated by viral infection and usually self-limited. In adult form is usually a chronic disease and only infrequently follows a viral infection.
Thrombocytopenic Purpura
Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU
Brief
1.definition：bleeding of skin、mucous and organs
2.cause： （1）production ：drug, tumor, infection,
radiation （2）destruction ：ITP,DIC,TTP （3）abnormal distribution：splenomegaly 3.classification：primary ,secondary
3.classification：acute, chronic
Mechanism
1.Immunity （1）acute：
postviral infection immune complex (viral Ag and Ab) connect with platelet Fc-R viral components banding with platelet auto Ab generation
（2）chronic：not affected by foreign Ag
Mechanism
2.liver and spleen：
（1）1/3 platelets are held within the spleen （2）spleen produces platelet associated Ab （3）liver and spleen erase platelet：7-11 days
• presenting complaint： mucosal or skin bleeding. Common types： epistaxis, oral bleeding, menorrhagia, purpura, and petechiae.
in normal persons, 1-3 days in patients
Mechanism
3.else：
（1）estrogen: ITP often seen in female patient hold down platelet production augment platelet clearance
Clinical Findings
• age ： It is a diHale Waihona Puke Baiduease of young persons, with peak incidence between ages 20 and 50
• sex： there is a 2:1 female predominance
Clinical Findings
• Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding, menorrhagia, gastrointestinal bleeding, purpura, and petechiae
• It is not clear which antigen on the platelet surface is involved.
• Although the antiplatelet antibody may bind complement, platelets are not destroyed by direct lysis
General Considerations
1.definition：thrombocyte unrelated to a drug, an infection, or autoimmune disease is generally classified as ITP
2.character： shortened span of platelet increased megakaryocyte in bone marrow
General Considerations
• Rather, destruction takes place in the spleen, where splenic macrophages with Fc receptors bind to antibodycoated platelets
• Since the spleen is the major site both of antibody production and platelet sequestration, splenectomy is highly effective therapy
Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU
General Considerations
• Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation)
IDIOPATHIC (AUTOIMMUNE) THROMBOCYTOPENIC PURPURA (ITP)
General Considerations
• ITP is an autoimmune disorder in which an IgG autoantibody is formed that binds to platelets
（2）more delicate capillary wall
Clinical Findings
• Symptoms and Signs: • occurs ：
in childhood, frequently precipitated by viral infection and usually self-limited. In adult form is usually a chronic disease and only infrequently follows a viral infection.
Thrombocytopenic Purpura
Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU
Brief
1.definition：bleeding of skin、mucous and organs
2.cause： （1）production ：drug, tumor, infection,
radiation （2）destruction ：ITP,DIC,TTP （3）abnormal distribution：splenomegaly 3.classification：primary ,secondary
3.classification：acute, chronic
Mechanism
1.Immunity （1）acute：
postviral infection immune complex (viral Ag and Ab) connect with platelet Fc-R viral components banding with platelet auto Ab generation
（2）chronic：not affected by foreign Ag
Mechanism
2.liver and spleen：
（1）1/3 platelets are held within the spleen （2）spleen produces platelet associated Ab （3）liver and spleen erase platelet：7-11 days
• presenting complaint： mucosal or skin bleeding. Common types： epistaxis, oral bleeding, menorrhagia, purpura, and petechiae.
in normal persons, 1-3 days in patients
Mechanism
3.else：
（1）estrogen: ITP often seen in female patient hold down platelet production augment platelet clearance
Clinical Findings
• age ： It is a diHale Waihona Puke Baiduease of young persons, with peak incidence between ages 20 and 50
• sex： there is a 2:1 female predominance
Clinical Findings
• Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding, menorrhagia, gastrointestinal bleeding, purpura, and petechiae
• It is not clear which antigen on the platelet surface is involved.
• Although the antiplatelet antibody may bind complement, platelets are not destroyed by direct lysis
General Considerations
1.definition：thrombocyte unrelated to a drug, an infection, or autoimmune disease is generally classified as ITP
2.character： shortened span of platelet increased megakaryocyte in bone marrow
General Considerations
• Rather, destruction takes place in the spleen, where splenic macrophages with Fc receptors bind to antibodycoated platelets
• Since the spleen is the major site both of antibody production and platelet sequestration, splenectomy is highly effective therapy